Behçet's disease with severe arterial involvement in a child

Clin Rheumatol. 2002 May;21(2):176-9. doi: 10.1007/s10067-002-8278-3.

Abstract

Behçet's disease is a vasculitis characterised by its thrombotic tendency. In some patients, manifestations of vascular lesions may dominate the clinical picture instead of the classic triad. We describe a 13-year-old boy with a 1.5-year history of Behçet's disease presenting with complaints of acute abdominal pain, severe headache and decreased vision. His work-up revealed a saccular aneurysm of the distal abdominal aorta, thrombosis in the right common iliac, external iliac and femoral arteries, and thrombosis of the superior sagittal sinus. Aortoiliac bypass with a Dacron graft was performed with success. He also received cyclosporin A and anticoagulant therapy. Severe vasculitis may become overt at any age in patients with Behçet's disease. Early diagnosis and management is important to prevent morbidity and mortality.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Angiography
  • Aortic Aneurysm, Abdominal / diagnostic imaging
  • Aortic Aneurysm, Abdominal / etiology*
  • Aortic Aneurysm, Abdominal / surgery
  • Arterial Occlusive Diseases / diagnostic imaging
  • Arterial Occlusive Diseases / etiology*
  • Arterial Occlusive Diseases / surgery
  • Behcet Syndrome / complications*
  • Behcet Syndrome / diagnosis
  • Blood Vessel Prosthesis
  • Femoral Artery*
  • Follow-Up Studies
  • Humans
  • Iliac Artery*
  • Intracranial Aneurysm / diagnostic imaging
  • Intracranial Aneurysm / etiology*
  • Intracranial Aneurysm / surgery
  • Male
  • Risk Assessment
  • Severity of Illness Index
  • Vascular Surgical Procedures